Sun-396 Renal Thrombotic Microangiopathy in Niemann-Pick Disease Type B: A Case Report

Abstract

Glomerulonephritis (GN) is one of the major causes of chronic kidney disease (CKD) worldwide. It remains the leading cause of End Stage Kidney Disease (ESRD) in many developing countries including Nigeria with varying clinical course and histologic pattern. The clinical outcome and the degree of renal impairment observed in patients with glomerulonephritides is dependent on the type and extent of the histological lesion identified in the glomerulus and tubulointerstitium. Renal biopsy remains an important diagnostic tool in patients with glomerulonephritis with varying indications. This study is unique because the histologic features observed in the glomerulus and tubulo-interstitium were graded, scored and correlated with clinical as well as biochemical features. This will add to the body of knowledge on glomerulonephritides in this environment. The objectives of the study was to determine the grading and severity of histologic features observed in the glomerulus and tubulo-interstitium and to determine the relationship (if any) between clinical, biochemical and histologic features in adults with GN at OAUTHC, Ile-Ife. Methods: The study was a cross sectional hospital based study of seventy ( 70) consecutive adult patients with features of glomerulonephritis who presented at the nephrology and other clinics of OAUTHC Ile-Ife. Renal function was assessed and renal biopsy performed after obtaining written informed consent. The renal tissues obtained were subjected to light microscopy and immunoperoxidase staining with IgA, IgM, IgG and C 3 antibodies and the degree of involvement of glomeruli, tubules, interstitium and vessels were graded from 1 to 5 for the glomeruli and 1 to 3 for the tubulo-interstitium respectively according to severity. Total activity and total chronicity indices were collated and their association/correlation with clinical parameters assessed.Results: A total of seventy patients participated and completed the study. Three (3) patients had inadequate renal tissue for histologic diagnosis hence data analysis was based on the remaining sixty seven (67) patients. Statistically significant correlations were found between the interstitial scores for activity (interstitial oedema, interstitial infiltrate) with serum creatinine and GFR: interstitial oedema with serum creatinine (r¼0.35, p¼0.003) and GFR (r¼-0.38, p¼0.004); interstitial infiltrate with serum creatinine (r¼0.52, p<0.0002) and GFR (r¼-0.70, p¼0.002) and total activity index with serum creatinine (r¼0.60, p¼0.0001) and GFR (r¼ -0.48, p ¼ 0.004). Statistically significant correlations was also seen between total chronicity index and test of renal function: total chronicity index with serum creatinine (r¼0.62, p¼0.001) and GFR (r¼ -0.58, p<0.00001). Conclusions: Both activity and chronicity indices significantly influence renal function. The higher they are the lower the GFR and vice versa, this suggests aggressive management protocol for patients with higher indices. The histologic scoring system would assist in assessing the severity of...